Introduction. Sudden
death during sports activities is not a rare incident with fatal
arrhythmia being the most common cause of death. Marfan syndrome is a
relatively common, preventable cause of sudden cardiac death in
athletes. It is an autosomal-dominant disorder involving the connective
tissue leading to aortic root aneurysm rupture or dissection. Symptoms
from the cardiovascular system include hyperventricular arrhythmia,
dyspnoia and mitral valve prolapse. Material. A
26-year-old man, a professional in gymnastics, presented to the
Emergency Room complaining of an acute onset of chest pain and back
pain and numbness of the left lower extremity. His past medical history
included hyperventricular arrhythmia and an incidence of automatic
bilateral pneumothorax four years ago. Results.The contrast
enhanced computed tomography revealed dilatation of the aortic root to
the level of the renal arteries; a Type I aortic dissection according
to the De Bakey Classification. The patient was submitted to the Bental
procedure but he unfortunately died during the cardiopulmonary bypass.
Marfan syndrome was diagnosed at autopsy. Conclusion. The Marfan
syndrome is usually diagnosed in young patients, younger than 35 years,
and is associated with a poor prognosis. Early diagnosis of the Marfan
syndrome is important so that cardiovascular complications be
prevented. Echocardiography is essential to rule out cardiovascular
involvement in those suspected of having Marfan syndrome. The
significant cost of large population screening should not put a limit
to the effort of clarifying the cardiovascular pathology responsible
for sudden cardiac death in young athletes. Key words: Marfan
syndrome, athlete, aneurysm, sudden death.